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Serial neuroimaging studies in Alexander's disease were obtained on an African-American girl who died at 4z\x years of age. She presented with macrocephaly, psychomotor retardation, spasticity, a seizure disorder, and hydrocephalus. A thorough metabolic evaluation of defined leukodystrophies, including Krabbe's disease, adrenoleukodystrophy, metachromatic leukodystrophy, Canavan's disease, and Leigh disease, was negative. A diagnosis of Alexander's disease was made based on the clinical features and ruling out all other possible causes. It was confirmed by pathologic findings of numerous subpial, subependymal, and perivascular Rosenthal fibers throughout the entire cerebrum. Interestingly, autopsy also identified the stenotic sylvian aqueduct owing to Rosenthal fiber accumulation, explaining the origin of hydrocephalus. The evolution of magnetic resonance imaging findings appears to be unique in this disease. (J Child Neurol 2002;17:463-466).

deviation of both eyes, absent oculocephalic responses, marked bifacial weakness, absent corneal reflexes, and bilateral tongue atrophy, as well as persistence of the previously noted hypertonia. Laboratory evaluation included normal computed tomography and magnetic resonance imaging of the brain. Sequential electroencephalogram examinations were appropriate for age, except for 1 study that showed a less mature pattern than expected. Electromyography and nerve conduction studies were normal. Ophthalmologic examination showed immature retina in zone 2 and exotropia. Brainstemevoked potentials performed between 11 and 12 weeks showed no transmission through the brainstem. Karyotype was normal female (46,XX). Because of the striking impairment in brainstem function, the diagnosis of Mobius syndrome was considered. The infant’s condition did not improve during a 3-month period. The neurologic condition was considered fatal regardless of underlying cause, and, after consultation with the infant’s parents, life support was withdrawn. The infant died at approximately 3 months of age. An unrestricted postmortem examination was performed.

BACKGROUND Opioid agonists can inhibit cell proliferation in various neural tumor cell lines, including rat gliomas. Because opioid antimitogenic effects are mediated by opioid receptors, it was of interest to the authors to determine opioid receptor levels in human brain tumors. METHODS Specimens obtained at craniotomy from 30 patients with glioma and nonneoplastic brain disorders were evaluated for their κ-opioid receptor binding. Kd and Bmax values were estimated from homologous competition binding curves with the κ1-selective radioligand [3H]U69,593. RESULTS Receptor binding density was greatest in nonneoplastic brain tissue, less in Grade 2 and 3 astrocytoma, and least in glioblastoma multiforme. CONCLUSIONS These results suggest that opioid receptor-based stratification of grade may have clinical utility in distinguishing glioblastoma multiforme from lower grade astrocytomas, and thereby may facilitate diagnosis and treatment. Cancer 1998;83:2561-2566. © 1998 American Cancer Society.

Background: Acidosis may contribute to brain injury from asphyxia, but its role is unclear. In order to evaluate the association between brain acidosis and cerebral injury, we subjected piglets to hypoxia and hypotension (HYP–HOTN) or hypoxia alone (HYP) to inflict varying amounts of brain damage. We hypothesized that piglets with a more severe brain injury would have a lower brain pH. Methods: Piglets had a pH microprobe inserted into the cerebral cortex. HYP animals breathed 5–8% O2/7% CO2 for 30 min with mean arterial pressure (MAP) maintained at >40 mmHg. HYP–HOTN animals breathed the same gas for 30 min, but during the last 15 min, MAP was reduced to 25–30 mmHg by withdrawing blood. After 4 h of recovery, the animals were perfusion-fixed and pathology assessed. Somatosensory-evoked potentials (SEP) were also monitored. Results: HYP–HOTN piglets had more neuropathology than HYP animals. During the last 15 min of injury, brain pH in the HYP–HOTN group was significantly higher than that in HYP. However, recovery of brain pH was prolonged in the HYP–HOTN animals. The amount of time for brain pH to recover to ≥7.00 correlated very well with both the degree of neuropathology and SEP recovery. The reduction in brain pH, either absolute or relative to baseline, was not associated with the severity of damage. Conclusions: The time needed for brain pH to recover after asphyxia, but not its severity, was associated with the amount of brain injury. Further study is warranted to determine whether immediate restoration of brain pH will reduce brain damage.

Background. Intramedullary neurosarcoidosis may be the first manifestation of the disease and may mimic a tumor clinically and radiographically. Two patients who presented with cervical intramedullary lesions on magnetic resonance imaging (MRI) scans were found to have neurosarcoidosis. Clinical presentation. Two patients with negative past medical history presented with progressive myelopathic features, and intramedullary cervical lesions were detected on MRI scan; the diagnosis was made on biopsy of the lesions. Early therapeutic intervention led to a favorable outcome. Conclusion. Intramedullary neurosarcoidosis, especially in the cervical cord, can be the initial presentation of the disease, mimicking a tumor. MRI scan, biopsy, and, in fewer cases, angiotensin-converting enzyme levels can help with the diagnosis and may lead to a favorable outcome.

We compared the effectiveness of transcutaneously activated magnetic distraction of an osteotomized cranial bone flap to surgical repositioning of the flap with immediate, rigid internal fixation. Thirty immature rabbits were studied. All 30 rabbits underwent complete circumferential osteotomies of both parietal bones and postoperatively all were fitted with head frames. The rabbits were divided into 4 groups. The experimental magnetic distraction group consisted of 10 animals (group 1). In these rabbits magnets were secured to both parietal bones and magnets of opposite polarity were placed in the headframes. Each morning, the magnets in the head-frames were moved 0.25 mm farther away from the skull; this continued for 20 days for a total setback of 5 mm. Five animals served as the magnetic distraction controls; in these animals magnets were placed on the parietal bones but none were attached to the headframes (group 2). The experimental surgical repositioning group consisted of 10 rabbits (group 3). In each the parietal bones were elevated and fixed 5 mm above the rest of the skull using vitallium mesh and screws. Five rabbits made up the surgical respositioning control group (group 4). In these animals, parietal osteotomies were performed but the bones were secured to the skull in their original positions using hardware identical to that used in the experimental group. Six weeks later all of the animals were sacrificed. Two significant differences were identified between the experimental groups: (1) the cranial contours of the animals in the magnetic distraction group were rounded while those of the surgically repositioned group were acutely angled; (2) the osteotomies in rabbits in the magnetic distraction group were essentially completely ossified while in rabbits in the surgically repositioned group there were obvious gaps at the osteotomy sites that were filled with fibrous tissue. These conclusions support the potential utility of magnetically activated distraction as an alternative to current methods of cranial vault remodeling.

The technique of distraction osteogenesis has not been widely used in the treatment of problems of the head and face because of the need for external fixators. By using magnetic, rather than mechanical, forces to drive bone movement we hope to expand the applications of the technique to include the treatment of cranial vault deformities. Thirty immature rabbits were studied. Twenty-six of the rabbits underwent operations. Each of the 26 had 2 magnets fixed to its skull: one on the left parietal bone and one on the right parietal bone. Incomplete circumferential osteotomies were then performed around each magnet on 13 of the rabbits. A head frame was attached to each animal. Head frames without magnets were placed on 6 of the rabbits (3 with osteotomy, 3 without osteotomy) while 20 of the animals (10 with osteotomy, 10 without osteotomy) had 2 magnets mounted on the frames which were of opposite polarity to those implanted. The remaining 4 rabbits served as nonoperative controls. Six weeks later all of the animals were sacrificed. There were significant differences in the cranial volumes and contours between the groups of animals. Many of the differences were increased by coincident osteotomy. Associated histologic findings are described.

A man developed spontaneous spinal epidural hematoma secondary to ruptured hemangioma with a clinical picture resembling acute partial Brown-Sequard syndrome. Diagnosis by magnetic resonance imaging (MRI) allowed prompt surgical decompression and complete reversal of the neurological deficits. The diagnosis, etiology, and MRI findings of spinal epidural hematomas are discussed.

The authors report a case of primary Ki-1 lymphoma of the brain. The patient was a 4 1/2-year-old black girl who presented with a 4- and 5-day history of headaches, nausea, vomiting, neck stiffness, and difficulty in walking. Computed tomography (CT) scan of the brain showed two discrete densities in the left occipital lobe and in the brain stem. Magnetic resonance imaging (MRI) showed multiple densities scattered over the brain surface and brain stem. Microscopically, the tumor was an anaplastic neoplasm that diffusely infiltrated brain parenchyma. The neoplastic cells were large with amphophilic cytoplasm, large nuclei with irregular nuclear contours and prominent nucleoli. A high mitotic rate including atypical mitotic figures was noted. Immunohistochemical stains showed diffuse strong positivity for CD30 and moderate focal staining for epithelial membrane antigen. Leukocyte common antigen, cytokeratin, neuron specific enolase, monocyte/macrophage and B- and T-marker stains were negative. The histology was characteristic for Ki-1 large cell lymphoma. Cytologic examination of cerebrospinal fluid (CSF) demonstrated similar neoplastic cells. This is one of the first reports of this variant in the pediatric population.

Noncontrast computed tomographic scans (CT scans) may show a hyperdense basilar artery before a brainstem infarct is visualized. This early sign should assist clinicians in confirming the diagnosis of basilar artery thrombosis. In a review of admission records of 750 patients with acute cerebrovascular disease from July 1991 to June 1993, at Saint Louis University Hospital, 20 patients were identified with clinical signs of nonlacunar, vertebrobasilar distribution infarction. Eight of these had pontomesencephalic ischemia. Their neuroimaging studies and medical records were evaluated. Four patients with acute clinical signs of pontomesencephalic infarction were found to have a hyperdense basilar artery on CT scans. The scans of 2 patients were excluded because of dolichoectasia; in the other 2 patients, the basilar artery appeared normal on the CT scan. The hyperdense basilar artery was detected within the early hours of neurological symptoms and often was the only detectable abnormality on the scan. In 3 patients extensive brainstem infarcts subsequently developed and they died. Basilar artery thrombosis was confirmed by pathological study in all these patients. In the fourth patient basilar artery occlusion and a large pontine infarct were evident by magnetic resonance imaging and angiography. A hyperdense basilar artery is a common feature on CT scans of patients presenting with an early clinical diagnosis of thrombosis. Untreated, the hyperintense basilar artery often portends a poor prognosis. Its ready recognition should guide further interventional studies and treatment.

A 72-year-old man had posturally induced syncopal episodes, followed by quadriparesis, coma, and death. Neuroimaging studies supported the clinical diagnosis confirmed by neuropathological findings.

Asphyxia causes acidosis which may potentiate neural injury. We hypothesized that hypoxia & hypotension cause lower brain pH and more severe neural injury than hypoxia alone. METHODS: Anesthetized piglets (age 0.05). Brain & serum pHs did not differ in either group (p>0.05). SEP recovery in HTN (6.3± 3.3% of baseline) was less than (p 0.05) over time. Brain and serum pH correlated (p